A hereditary autosomal dominant immunologic deficiency syndrome characterized by NEUTROPENIA; AGAMMAGLOBULINEMIA, and extensive human papillomavirus (HPV) infection. Despite the peripheral neutropenia, bone marrow aspirates from affected individuals contain abundant mature MYELOID CELLS (myelokathexis). The susceptibility to HPV is disproportionate compared with other immunodeficiency conditions. Mutations in the CXCR4 gene have been identified. OMIM: 193670
